Prospective Study of Fibrosis In the Lung Endpoints (PROFILE - Central England)

Prospective Study of Fibrosis In the Lung Endpoints (PROFILE - Central England)

The overall aim of this study is to develop a test that predicts the prognosis of IPF (Idiopathic Pulmonary Fibrosis) and which could be used to determine whether new treatments for IPF are likely to work.

The overall objectives of this study are

• Discover and validate novel biomarkers and gene expression profiles for use in subsequent intervention studies in patients with IPF
• To prospectively validate a panel of previously published biomarkers in patients with well characterized idiopathic fibrosing lung disease
• Investigate genetic associations and epigenetic modifications which affect disease severity and progression
• Prospectively evaluate longitudinal disease behaviour in patients with IPF and NSIP with a view to developing composite clinical end-points for subsequent use in intervention studies in patients with IPF

Biomarkers that can be used for the following purposes will be identified:

• Identify patients (Diagnostic)(e.g. discriminate between health and disease)
• Correlate with disease severity (extent of disease, staging of disease)
• Predict clinical progression (Prognostic)(stable vs progressive disease)
• Track response to therapy (Therapeutic response)- Predict response to known efficacious treatments & Correlates with changes in clinical endpoints/mortality/quality of life
• Predict risk of exacerbations (could be used to prevent exacerbations or reduce their severity)
• Correlate with complications and/or comorbidities (e.g. biomarkers of Pulmonary Arterial Hypertension, Gastro Oesophageal Reflux in IPF, etc)

Inclusion Criteria:

A diagnosis of IPF using the consensus criteria (32)and Non Specific Interstitial Pneumonia.

• Between the age group 18-85 years.
• Sub classified into Mild (TLCO>60), Moderate (TLCO 40-60), Severe (TLCO<40).
• People who volunteer to undergo a bronchoscopy for research

Exclusion Criteria:

• People who do not have IPF/NSIP (i.e. Hypersensitivity Pneumonitis, Sarcoidosis)
• People who cannot give informed consent.
• People who are being considered for bronchoscopy, any contra-indication to undergoing this procedure as set out in the British Thoracic Society guidelines (Thorax 2001; 56: suppl I: i1-i21). These will be part of the study but not undergo the Broncho Alveolar Lavage.